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  • Title: Dietary therapy in two patients with vitamin B12-unresponsive methylmalonic acidemia.
    Author: Satoh T, Narisawa K, Igarashi Y, Saitoh T, Hayasaka K, Ichinohazama Y, Onodera H, Tada K, Oohara K.
    Journal: Eur J Pediatr; 1981 Feb; 135(3):305-12. PubMed ID: 7227387.
    Abstract:
    The biochemical and therapeutic responses to dietary therapy were studied in a 25-month-old girl and a 1-month-old girl with methylmalonic acidemia (MMA-emia), which was unresponsive to vitamin B12. The minimum daily intake of protein which patients could tolerate and display a good development was between 1.0 and 1.2 g per kg body weight. Supplementation with amino acid mixture devoid of toxic amino acids was required to prevent protein malnutrition when daily protein intake was restricted to 0.6 g per kg body weight. Caloric intake should be sufficient, not only to promote growth but also to prevent a rise in MMA level, especially when a patient has ketoacidosis. It was found that MMA excretion per mg creatinine in random urine specimens correlated significantly with serum MMA and twenty four-hour output of MMA per kg body weight. Therefore measurement of MMA in a single urine specimen is useful for evaluating the in vivo accumulation of MMA.
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